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At least 19 recordsLinked to original sources
PubMed1997

Encephalopathy due to capillary leak syndrome.

Systemic capillary leak syndrome (SCLS) is characterized by intermittent attacks of leakage of intravascular fluids into the extravascular space. Hypovolemia, hemoconcentration, weakness, edema, and visceral congestion are resulting manifestations of SCLS. Most patients with SCLS have clear mentation during attacks, and encephalopathy is not a known manifestation of the syndrome. We report a patient with acute idiopathic capillary leak syndrome manifested in an acute encephalopathy. The possibility of SCLS should be considered in patients who have an encephalopathy and hemoconcentration.

Brain Diseases↗
PubMedDate not supplied

[The clinical relevance of subcutaneous-thoracic ratio in preterm newborns as a possibility for quantification of capillary leak syndrome].

BACKGROUND: Capillary leak syndrome (CLS) is associated with increased morbidity and mortality in preterm newborns (PN) with sepsis or necrotizing enterocolitis. PATIENTS AND METHODS: In order to provide improved standards for measuring edema and for the definition of CLS, subcutaneous-thoracic ratios (S/T) were calculated from 821 anteroposterior supine chest radiographs of 119 PN with a birth weight below 1500 g. The S/T was computed by 100 % minus ratio of dividing the outer margins of the eighth rib by the total diameter of the thorax at the same position. Birth weight, gestational age, need for assisted ventilation and the position of the diaphragm did not significantly affect S/T. The S/T drifted downwards slightly with postnatal age. RESULTS: To provide normal standards of S/T in PN, the ratio was calculated in percentiles and the higher percentiles correlated with diseases. 771 S/T were lower than 10.1 % (95th percentile), 21 were 10.1 - 12.6 % (97.5th percentile) and 20 were even higher. 3 of the 100 PN (84 %) with an S/T < 10.1 % died because of extreme immaturity. Eight patients (6.7 %) had an S/T between the 95th and the 97.5th percentile in at least one of their radiographs. Four of them had a respiratory distress syndrome and one died. Eleven PN had an S/T > 12.6 %. All of them showed a multiple organ failure and four died. When the SIT was > 15 % the edema became visible. A CLS was diagnosed in two PN with an S/T > 20 %. CONCLUSIONS: The threshold for the capillary leak syndrome was found to be 12.6 % also in reference to the course of diseases. The S/T is a useful tool because it is simple to measure and calculate, and is available from a single frontal film. The ratio can measure objectively the edema and the CLS in PN.

Capillary Leak Syndrome↗
PubMed1996

Marrow engraftment and clinical manifestations of capillary leak syndrome.

A capillary leak syndrome occurs frequently in bone marrow transplant patients in addition to graft-versus-host-disease and infection. The underlying pathology is poorly understood but the clinical manifestations of excessive weight gain, ascites, and edema associated with kidney and liver abnormalities suggest a common injury to multiple organs. The clinical courses of 55 allogeneic and autologous marrow transplant recipients were retrospectively reviewed with regard to the presentation of a capillary leak syndrome (CLS). Twenty-nine patients (53%) developed non-cardiogenic pulmonary edema with or without concurrent pleural effusions; the incidence was comparable in allogeneic and autologous recipients. Pulmonary features were accompanied by hepatic dysfunction in 28, renal dysfunction in 22, and central nervous system abnormalities in 17. There was a strong correlation between time of engraftment and the first manifestations of CLS, both of which occurred earlier in allogeneic than in autologous transplant recipients. These data confirm that CLS occurs in both allogeneic and autologous transplant recipients and suggest a pivotal contribution by circulating leukocytes.

Bone Marrow Transplantation↗
PubMed1995

Systemic capillary leak syndrome.

Systemic capillary leak syndrome is a rare, severe disorder with a high mortality rate. It consists of the shift of fluid and proteins from the intravascular to the extravascular compartment with subsequent hypovolemic shock. We describe a 34-year-old-woman who had several episodes of generalized edema that evolved to hypovolemic shock. During the acute phase, laboratory investigations revealed marked hypoproteinemia, leukocytosis, and high levels of hematocrit and hemoglobin. A paraprotein IgG kappa chain was detected. Although different therapeutic trials were used, the patient continued to have similar episodes and she died during an acute episode 2 1/2 years after the first symptom of this disorder. The cause of systemic capillary leak syndrome is unknown. The presence of a paraprotein IgG is frequent in this group of patients.

Adult↗
PubMed2001

Gemcitabine-induced systemic capillary leak syndrome.

Systemic capillary leak syndrome (SCLS) is a rare disorder with a high mortality rate, characterized by rapidly developing edema, weight gain and hypotension, hemoconcentration and hypoproteinemia. This syndrome is caused by sudden, reversible capillary hyperpermeability with a rapid extravasation of plasma from the intravascular to the interstitial space. Even though SCLS has been suggested to be the pathogenic mechanism for the pulmonary toxicity of gemcitabine (GCB), a new deoxycytidine analogue with structural similarities to cytosine arabinoside, a direct correlation between GCB and SCLS has never been reported. We describe a case of repeated SCLS after GCB administration in a 51-year-old male with locally-advanced non-small-cell lung cancer treated with a combination of cisplatin and GCB. The detection of GCB-induced SCLS supports the hypothesis that SCLS could be the pathogenic way of GCB pulmonary toxicity. This finding can help to better understand and treat the potentially deadly GCB-related acute respiratory distress syndrome that is being recognized.

Antimetabolites, Antineoplastic↗
PubMed2002

Systemic capillary leak syndrome.

Systemic capillary leak syndrome (SCLS) is characterized by recurrent hypovolemic shock attributable to increased systemic capillary leakage. A 46-year-old man was admitted to our hospital because of recurrent episodes of generalized edema with hypovolemic shock. Blood laboratory data revealed severe hypoproteinemia with a small monoclonal IgG-kappa protein. These findings suggested strongly the diagnosis of SCLS. A regimen with steroid pulse therapy was tried; however, intravascular overloading accompanied by the recruitment of the initially extravasated fluids resulted in acute pulmonary edema. Intensive care and careful monitoring of fluid volume are required in SCLS.

Capillary Leak Syndrome↗
PubMed2005

[Refractory oedema: think about systemic capillary leak syndrome].

Systemic Capillary Leak Syndrome (SCLS) is a rare and recently discovered disease. The diagnosis must be evoked in front of an hypovolaemic shock associated with a refractory anasarca. We report an observation of SCLS and then discuss the diagnostic and therapeutic difficulties. SCLS is diagnosed when a patient meet a compatible clinical situation with an hypoalbuminemia in spite of relative hemoconcentration. Evidence of a monoclonal gammapathy constitute an additional clue.

Aged↗
PubMed1993

The paraproteins in systemic capillary leak syndrome.

Systemic capillary leak syndrome (SCLS) is a rare disease characterized by episodes of collapse due to rapid transfer of considerable volumes of plasma from the intravascular to the extravascular compartment. The pathogenesis of this disease is unknown. The diagnosis is made largely on clinical grounds, and investigations are unhelpful. The only consistent abnormality is that an IgG paraprotein is found in most patients, raising the possibility that the paraprotein may be involved in the pathogenesis of the disease. Reduction of the paraprotein level in our patient was associated with remission. Blood samples from three SCLS patients and one probable SCLS have been studied. All patients had monoclonal IgG paraproteins. The purified paraproteins were all of IgG1 subclass and had kappa light chains. However, they differed in size and charge. Antibodies against each of the paraproteins were raised in rabbits. Affinity-purified anti-idiotypic antibodies were tested for cross-reactivity against the other paraproteins using immunoblotting and Ouchterlony assay. These assays showed that the anti-idiotypic antibodies reacted only with the immunizing paraprotein and not with any of the other paraproteins, i.e. that the paraproteins do not share a common idiotype. Paraproteins did not bind to cultured endothelial cells, either unactivated or following activation with interferon-gamma (IFN-gamma), IL-2 or IL-6. In addition, we were unable to demonstrate any cytotoxicity towards cultured human endothelial cells by paraprotein alone, or in the presence of neutrophils (pronounced neutrophilia being a feature of attacks). The relationship between the paraproteins and the disease remains unclear. It is likely that additional, as yet unidentified, factors are required for the paraprotein to lead to capillary leak.

Blood Protein Electrophoresis↗
PubMed1990

Systemic capillary leak syndrome.

Systemic capillary leak syndrome is a rare condition characterized by unexplained episodic capillary hyperpermeability. A shift of fluid and protein from the intravascular to the interstitial space results in hypovolaemia. Attacks vary in frequency, severity and duration and can be fatal, although they often remit spontaneously. During acute episodes there is a pathognomonic elevated haematocrit and reduced serum albumin, frequently associated with a monoclonal gammopathy. Treatment has been largely empirical but there are anecdotal reports of beneficial therapy. We describe a further case which highlights the typical clinical presentation, course and investigate findings and review the other cases described.

Blood Volume↗
PubMedDate not supplied

Activation of the complement system during immunotherapy of cancer with interleukin-2: a possible explanation of the capillary leak syndrome.

The capillary leak syndrome, responsible for fluid loss into the interstitial space, represents one of the major cardiovascular toxicities of IL-2 during the immunotherapy of cancer. The mechanisms involved in the increased vascular permeability have still to be better understood. The present study was carried out to investigate the role of the complement system in mediating the IL-2 vascular toxicity. The study was performed in metastatic renal cancer patients, treated with IL-2 through a 24-hour i.v. infusion at a daily dose of 3 x 10(6) U/m2 for 5 consecutive days, corresponding to one IL-2 course. Six IL-2 courses were evaluated. C3 and C4 were measured daily during IL-2 infusion, and 2 and 5 days after its interruption. IL-2 administration induced a significant decrease in both C3 and C4 mean levels, which became within the normal range 5 days after the end of IL-2 infusion. These results show that IL-2 administration may directly activate the complement system through the classical pathway, which might play a role in determining the increased vascular permeability.

Capillary Permeability↗
PubMed1994

Biclonal Sézary syndrome with capillary leak syndrome.

Sézary syndrome is a rare, usually monoclonal proliferation of T lymphoid cells. We report a case of this form of cutaneous T cell lymphoma with highly probable biclonality of neoplastic proliferation. Biclonality in malignant lymphoma is a rare feature, until now generally reported in B cell lymphoma. Its identification by accurate immunological studies could be of importance since one of the two populations can exhibit a less differentiated phenotype requiring a more aggressive therapeutic regimen than usual. The occurrence of anasarca in our patient was probably due to capillary leak syndrome, already discussed in another case of peripheral T cell lymphoma.

Aged↗
PubMed2001

Initial evidence of endothelial cell apoptosis as a mechanism of systemic capillary leak syndrome.

BACKGROUND: Systemic capillary leak syndrome (SCLS) is a rare disorder of unknown etiology that is characterized by acute recurrent attacks of hypovolemic shock commonly following an inflammatory stimulus such as a viral illness. Prophylactic therapy is generally ineffective, and the outcome is frequently fatal. METHODS: In order to investigate the cellular mechanisms leading to SCLS, we examined the effects of sera from two patients with active SCLS on microvascular endothelial cell apoptosis in vitro. Apoptosis was determined by morphologic criteria, DNA fragmentation, annexin V stain, and by a quantitative photometric assay. The apoptotic pathway was investigated by Western blot of endothelial cells lysate after exposure to SCLS sera. RESULTS: The sera from patients with active SCLS mediated profound apoptosis of microvascular endothelial cells shortly after exposure. The exposed microvascular endothelial cells underwent immediate apoptosis as evidenced by morphologic changes, plasma membrane phosphatidylserine exposure, and by DNA fragmentation. Increased Bax/Bcl-2 ratio in endothelial cells exposed to SCLS sera was observed and suggested an oxidation injury as the possible mechanism for endothelial apoptosis. This potential mechanism was further explored by measuring intracellular reactive oxygen species (ROS) following SCLS serum exposure. Sera from both patients caused marked increases in ROS, initially detectable at 1 h and persisted for at least 12 h, with control serum from healthy subjects showing no effect on basal endothelial cell ROS concentrations. CONCLUSION: Components from the sera of patients with active systemic capillary leak syndrome in contrast to healthy subject sera mediate early and extensive endothelial apoptosis in vitro that is associated with oxidation injury. These data represent compelling initial evidence for oxidation-induced apoptosis as a likely mechanism for endothelial injury leading to SCLS.

Apoptosis↗
PubMed1997

[Systemic capillary leak syndrome].

The systemic capillary leak syndrome is a rare condition, with a high mortality rate, characterised by recurrent episodes of generalized edema, with hemoconcentration and hypoproteinemia, associated with paraproteinemia. Pathophysiologically, this syndrome is caused by a sudden, reversible increase in capillary permeability, with a rapid shift of plasma from the intravascular to the extravascular compartment with subsequent hypovolemic shock. We report a new patient of this unusual condition, and we review its diagnostic and therapeutic aspects.

Capillary Leak Syndrome↗
PubMed1996

Acute renal failure, compartment syndrome, and systemic capillary leak syndrome complicating carbon monoxide poisoning.

CASE REPORT: Five Indian labourers sharing one room were found lying on the floor unconscious. A coal fire had been lit to provide heat during the cold night and was still burning inside the room. Two of the men were pronounced dead at the scene. The other three were transferred to the hospital in critical condition. This paper contains the case reports of the three patients who were transferred to the hospital. One of the patients died 60 h after admission. The other two patients were successfully treated and released. The three patients developed severe rhabdomyolysis complicated by compartment syndrome. The patient who died developed compartment syndrome in three extremities. Two patients required blood transfusion due to very severe hemolysis. The hemolysis in the third patient was less severe and did not require blood transfusion. Two patients developed anuric acute renal failure due to acute tubular necrosis as shown by renal biopsy. Two patients developed the full blown picture of systemic capillary leak syndrome. Gross and microscopic examination of specimens taken at autopsy showed extensive necrosis of all skeletal muscles, myocardial necrosis and acute tubular necrosis affecting both kidneys.

Acute Disease↗
PubMed1998

Soluble L-selectin plasma concentrations in infants undergoing heart surgery: no association with capillary leak syndrome or need for respiratory support.

OBJECTIVE: In critical care patients at risk of developing the acute respiratory distress syndrome (ARDS), low soluble L-selectin (sCD62L) plasma concentrations have been shown to be associated with progression to ARDS and prolonged subsequent mechanical ventilation. This study aimed to determine the usefulness of sCD62L plasma concentrations to identify infants undergoing cardiovascular surgery who are at risk for postoperative pulmonary dysfunction and capillary leaks. DESIGN: Serial measurements of sCD62L plasma concentrations in a cohort of infants with congenital heart disease before, during, and after surgery for 4 consecutive days. SETTING AND PATIENTS: Infants aged 3-337 days undergoing cardiovascular surgery with (N = 27) or without (N = 12) cardiopulmonary bypass in a tertiary care center. RESULTS: sCD62L concentrations before surgery showed a strong correlation with the infant's age (r = 0.77, p < 0.001). During surgery, sCD62L levels dropped from 9.0 +/- 0.7 to 5.6 +/- 0.4 nmol/l (mean +/- SEM; p < 0.001). The minimum sCD62L concentration during and after surgery did not differ between infants operated upon with or without cardiopulmonary bypass (p > 0.1) or in infants who did (N = 10) or did not (N = 29) develop capillary leak syndrome. Whereas capillary leak syndrome was associated with prolonged mechanical ventilation (p < 0.01), there was no relationship between sCD62L concentrations at baseline or at any time thereafter and number of hours on the ventilator(p > 0.1). CONCLUSION: sCD62L concentrations before or after surgery are not apt to identify infants at increased risk of prolonged mechanical ventilation.

Age Factors↗
PubMed1996

[Capillary leak syndrome disclosing Ofuji's papuloerythroderma].

INTRODUCTION: Capillary leak syndrome is a specific entity among syndromes with capillary hyperpermeability. Endothelial cell activation is related to the higt level of adhesion molecules (sICAM-1, sVCAM-&, sCD62E) possibly due to several cytokines (IL-2, TNF ...). CASE REPORT: An 84-year-old woman was hospitalized for erythroderma. Ofujui papuloerythroderma was diagnosed and edema was attributed to capillary leak. A kinetic study of several cytokines and adhesion molecules sCD62E, sVCAM-1 and sICAM-1 was done. Outcome was favorable with corticopuvatherapy. DISCUSSION: The capillary leak syndrome reported here is simlar to that described in other erythrodermas with or without lymphoma. The keratinocyte would be activated by the CD4 T lymphocyte via the gamma-interferon mediator. The T cell secretes cytokines (interleukin-1, tumor necrosis factor ...) which activates the endothelium and increases vascular permeability. The level of adhesion molecules and changes observed during the episode of edema demonstrated the endothelial activation.

Aged↗
PubMed1987

Production of 5-lipoxygenase pathway metabolites by peripheral leucocytes in capillary leak syndrome (Clarkson disease).

Periodic systemic capillary leak syndrome (Clarkson disease) is characterized by unexplained attacks of a marked increase in capillary permeability. As leukotrienes, derived from arachidonic acid via the 5-lipoxygenase pathway, enhance capillary permeability, we studied arachidonate metabolism in leucocytes of a patient with capillary leak syndrome. Leucocyte-platelet suspensions, prepared from blood collected from the patient during asymptomatic periods (n = 11) produced greater amounts of 5-hydroxyeicosatetraenoic acid (5-HETE) than control suspensions (P less than 0.05). Peripheral leucocytes, collected during attacks (n = 3) and studied without addition of A23187 released LTB4 in vitro but not sulphidopeptides leukotrienes. This result was never observed with leucocytes from control subjects or from the patient out of a crisis. These results suggest that in the patient, peripheral leucocytes could be stimulated by an unknown, as yet to be determined, endogenous factor to produce more 5-HETE and LTB4. Whether LTB4 plays a pathogenic role in the capillary leakage remains to be determined.

Adult↗
PubMed2003

Decreased hepatic uptake of gallium-67 citrate in haemophagocytic syndrome occurring concomitantly with capillary leak syndrome.

We describe a 4-year-old girl with haemophagocytic syndrome (HPS) in whom hepatic gallium-67 citrate (Ga-67) uptake was suppressed when the disease was in its acute phase and returned to normal when the disease was in remission. The prominent clinical feature of this case was the occurrence of systemic capillary leak syndrome (CLS). Because extravasation of plasma proteins may be the result of vascular endothelial injury in CLS, loss of hepatic Ga-67 uptake may reflect insult to the hepatic sinusoidal endothelium. This case suggests a possible role of sinusoidal endothelial cells in the mechanism of hepatic Ga-67 uptake and indicates the need for further study of Ga-67 uptake in patients with HPS.

Capillary Leak Syndrome↗